About Me

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

The issue arose after South Korea agreed this April to lift most of the restrictions it had placed on U.S. beef imports. That prompted intense protests by South Koreans who say they fear mad cow disease in U.S. beef. They want their government to negotiate a tougher deal or to scrap it.

"Every single carcass that's processed is inspected by a USDA inspector," Schafer told reporters in San Antonio. "That beef is stamped A-OK, and we want to assure our consumers here in the United States, as well as our consumers ... in foreign countries, that we provide a good, clean, safe, abundant food supply here."

But what exactly is entailed in that inspection? According to the USDA, a government inspector is on site whenever cows are slaughtered and processed. The inspectors are supposed to look at every carcass and determine whether the meat is fit for human consumption. Basically, they have a look and maybe a sniff and a feel. That's it.

But even that cursory process might be more than consumers are actually getting. The Web abounds with reports, including firsthand accounts and interviews with reputable news organizations, in which USDA inspectors complain that they can't possibly carry out their job in a meaningful way. There are too few of them to deal with the number of cattle slaughtered each hour in modern meat-processing facilities.

The speed with which cattle are killed, skinned and cut up in these plants makes the job dangerous for the meat processors, to say nothing of inspectors who attempt to get close enough to a side of beef for a poke and a sniff. The high speed of operations sometimes does not allow cows to be properly stunned and bled to death by the time the skinning and cutting begins. That's not only cruel and inhumane, but also detrimental to food safety. Struggling animals mean meat falling on filthy floors, improper evisceration that spills feces onto meat and greater opportunities for cross-carcass contamination.

The shortage of inspectors also means that a USDA employee cannot always be available to inspect animals before they are killed to ensure that so-called downer cows are not processed. Cattle that cannot walk into the slaughterhouse because they are diseased or injured are more likely to be animals that carry bovine spongiform encephalopathy, commonly known as mad cow disease.

In February, the Humane Society of the United States released videotapes showing meat workers shocking nonambulatory cows, bumping them with forklifts and otherwise abusing them to force them onto their legs long enough to be certified for slaughter.

That's why many American consumers are voting with their pocketbooks for better meats. They are turning to local farmer's markets for cruelty-free meats from pasture-raised animals, forgoing meat from industrially raised cows, chickens and pigs that spend their lives packed into filthy cages, fed unhealthy diets and pumped full of antibiotics and hormones.

Increasingly available at local farmer's markets is beef from cows that are butchered humanely and in small numbers. As one farmer at Houston's Bayou City Farmer's Market put it one recent Saturday morning, "These are cows who have just one bad day."

Given the alternative practiced in processing plants, it's no wonder many foreign buyers of U.S. meat products are skeptical. Industrial beef producers employ practices that can be, in a word, repulsive. Until 1997, the United States permitted feeding cattle on beef waste products. It tested very few animals for mad cow disease, even though Europe was testing 10 million of its cattle each year, and the Japanese were testing each one. USDA allowed downer cattle into the food supply, a practice now banned. A 2004 ban on feeding cow's blood mixed with formula to calves and chicken droppings to cows was never put into practice.

According to The New York Times, the Agriculture Department has been fighting a lawsuit from a Kansas beef producer over the department's refusal to allow it to test for mad cow disease so that the producer can resume beef shipments to Japan.

None of this is reassuring. Instead of spouting empty rhetoric that U.S. beef is "the safest in the world," the USDA owes it to consumers to guarantee that meat meant for their dinner plates is processed without unnecessary cruelty and with standards that will produce a clean product that's safe to eat.

THE Korean beef market, once the third-largest importer of American beef, has shut its doors to the United States. Why? Because Koreans are worried about eating meat tainted with mad cow disease, which can be fatal to humans. Recent attempts by Korea’s president, Lee Myung-bak, to reopen the market have brought tens of thousands of demonstrators to the streets in protest.

American beef producers could easily allay those fears by subjecting every cow at slaughter to the so-called rapid test, which costs about $20 per carcass and screens for this brain-wasting disease in a few hours rather than days. But the United States Department of Agriculture won’t allow that.

In 2004, Creekstone Farms in Arkansas City, Kan., wanted to test the cattle it slaughters to comply with the wishes of its Korean and Japanese customers. But the department ruled that the rapid test could only be used as part of its own mad cow surveillance program, which randomly checks about 1 in 1,000 dead and slaughtered cattle in the United States every year. The sale of the kits to private companies is prohibited under an obscure 1913 law that allows the department to prohibit veterinary products that it considers “worthless.”

Creekstone sued the government in 2006, arguing in court that the Agriculture Department could not deem worthless a test that it used in its own surveillance program. The court agreed, but the department appealed. A decision is expected soon.

It is hard to understand why the Agriculture Department wants to stand in the way. Yes, the test has limitations: it can miss a case of mad cow disease, also called bovine spongiform encephalopathy, in the very early stages of incubation. But it can catch the disease in later stages, before animals show symptoms. Between 2001 and 2006, the European Union used the test to turn up 1,117 cases of mad cow disease in seemingly healthy cattle approved for slaughter.

Ideally, the Agriculture Department would follow the rules set up in Europe and Japan that require every cow over a certain age to be tested before being slaughtered. At the very least the department should not prevent private companies from testing.

Companies that use the rapid test should also be allowed to label their meat as having been “tested for mad cow” for American consumers who would like this extra level of protection. A Consumers Union national survey done in January 2004 found that 71 percent of adults who eat beef would pay more to support testing, and of those, 95 percent were willing to spend 10 cents more per pound for tested meat.

In the Creekstone case, the Agriculture Department argued that the tests should be prohibited because if one company started using them, consumer demand would drive all companies to use them, and that would add to the price of beef. But would that be such a bad thing? Isn’t this how the laws of supply and demand are supposed to work?

Most Americans, like Koreans, understand that testing for mad cow could save lives — and they’d like to have that option.

Beef Imports to Korea: An Open Letter to President Bush Korean middle school student Chae-song Kim asks that the trade agreement be reconsidered

Chae-song Kim (internews)

Published 2008-06-14 17:04 (KST)

Dear Mr. President,

Hello. I am an ordinary Korean teenager but I am not sending you this letter for an ordinary reason. I wanted to talk about something very serious with you. It is about the new agreement between the Republic of Korea and the United States.

Our countries are now both very sensitive about the beef issue. My belief is that free trade must be fair trade, as US ambassadors are on record as saying in the FTA between Peru, which means that both countries should benefit.

I admit that Korea has benefitted from trade in the areas of automobile exports, IT products, and cell phones. However, Koreans are very upset with their government. This is clear from the candlelight demonstrations in Korea, yet our government is saying that it is not possible to renegotiate the KORUS FTA agreement because of the federal government.

This is a photo that I took from the Seoul Plaza Hotel. There were more than 700,000 people gathered there. The news reported that only 19.7 percent said "yes" to President Lee. The rule about the trade agreement says, roughly, that within 20 days of the signing, if many citizens say "no" to the agreement, it is possible to make changes. What would be the problem to change the agreement now?

If the US and the Korean government do not accept people's opinions, the next generation of Koreans will have a negative image of the United States. We are trying not to buy products made in the United States and we do not respect our President. In the long term, demonstrations by Koreans will cause more damage to the US than changing this agreement would.

I've read that on March 20, 1996, BSE (Bovine Spongiform Encephalopathy) spread in the UK because prions are not destroyed until heated to 600 degrees Celsius.

This tells us that there is a high possibility of BSE infection in humans because even if humans cook their beef products thoroughly, prions are not going to be destroyed. Even the US Congress is saying that Canadian beef is dangerous and that the US shouldn't import Canadian beef aged over 30 months.

Even if Congress still says that Koreans have no proof about the dangers of BSE in US beef, we should err on the side of caution and emphasize prevention. Why would the European Union say "no", completely, to US beef? Why would the US itself not consume beef older than 30 months? The truth is that there is a possibility of it being very dangerous.

BSE has spread since the 1980s. Scientists say that it is caused by the use of animal feed made from cows that already have the disease. Since then, for 20 years, in countries all around the world, cows in countries like the United Kingdom, have consumed such feed and BSE became a serious problem. Along with Europe, in 2001 Japan detected BSE in cattle and in 2003, even the US discovered BSE in its stocks.

Regardless of this, I've been told that the United States is still allowing the production of feed based on cow parts. Also, without any outstanding symptoms, the cow is butchered and allowed to be exported. With this in mind, would Korean citizens feel safe about imported US beef?

I want to ask you: Wouldn't this agreement be beneficial to the US only in the short term? Couldn't this agreement be perceived as violating the rights of Koreans to feel safe? Please consider the result that this agreement could and will leave, both now and for the future.

I am considering going to an Ivy League college. If I succeed, and even if I don't, children in the States are my friends. Their family is my family. I just want my family in Korea and my US family to be healthy. I hope you’ll be remembered as a president who cared for citizens' health.

Believe in what your heart is telling you. Your Countryman and Countrywoman should be proud of your stance to protect your people. Please continue your fight for the truth, as the truth will set us all free. ...TSS

well, so the U.S. slams the mad cow door on Korea and it's people, due to the bungled beef deal the USDA shoved down Lee's throat. sadly, Lee signed the deal oblivious to what really has been going on behind closed doors for years here in the USA, and the USDA et al knew they had a fish on the line. my God, this guy was totally ignorant of what they were doing. now the kind honorable people of Korea will be force fed USDA certified beef. beef that has been highly suspect of mad cow disease since the USDA shut down testing, this after finding two cases of the atypical BSE in Alabama and Texas. remember, atypical BSE is more virulent than the UK BSE strain. Also, it seems the O.I.E. has sealed the deal on trading all strains of TSE i.e. mad cow disease strains globally, all for a buck, commodities and futures, to hell with human health. it's business as usual folks, eat up, and die old and demented, if your lucky. I must apologize to the kind honorable citizens of Korea for what my Government has done. I tried. But as the USDA certified beef starts to flood Korean markets, remember one thing Korea, you don't have to buy it. let it rot, until the USDA et al gets there head out of their pockets, and start to test all food producing cattle and all livestock for BSE and all TSE. CJD is a slow death while incubating. so you will not see the body bags all at once. as in the past here, it will be labeled as dementia, misdiagnosed as Alzheimer's and other dementia ailments. it will become an acceptable death as here in the USA due to the push by the industries and your Government due to the financial aspect of it. The OIE and my Government sold their souls to the devil, and if you don't believe me, just read the history. let it all be sporadic and or spontaneous they say, and make them eat it, and like it. that's their motto. to hell with the consumer. Every American and Korean consumer should be demanding 100% BSE/TSE mad cow testing on all livestock food producing animals, for humans and animals. This should be a no-brainer, but instead, it's a brain eater. Consumption of beef is but only one route of many, that can kill you from mad cow disease (all strains). friendly fire i.e. iatrogenic CJD from the medical and surgical arenas, dental, and blood, all are a real threat, from 2nd, 3rd, 4th passage, from someone that consumed meat from an animal with TSE. They can be long incubators, not clinical yet, but they can go on and infected many more via these routes, so please do not get hung up on the 'hamburger only' route. this is one of many routes, from one of many strains, from only one species. WE must take all Transmissible Spongiform Encephalopathies as the real threat they really are. DO NOT let the incubation period of all these documented TSEs in cattle, sheep, goats, deer, elk, mink, cats, do not let them fool you. The long term threat is very real, it's been real, and it's been ignored, all the while CJD is rising in the USA. all the time the USA has been shipping cattle and feed to God knows whom. again, it was the OIE, the USDA et al, and their BSE MRR (Minimal Risk Region) rule, that opened the gates to the exporting of all strains of TSE globally. This new rule set back the eradication BSE to the stone age, or back to day one in or around 1985, when BSE was first _documented_. The BSE MRR policy erased all attempted eradication of BSE. This will be one more of GWs et al sad, sad legacies that will go down in history as nothing more than what the UK did back when they failed to warn the world of their tainted cattle, MBM (greaves) etc. they just continued to ship it around the globe. BUT what the OIE, GW, and the USDA did was simply made it legal i.e. BSE MRR, the legal trading of all strains of TSE globally $$$

Wednesday, June 11, 2008

OIE Recognition of the BSE Status of Members RESOLUTION No. XXI (Adopted by the International Committee of the OIE on 27 May 2008)

Why Americans, As Well as Koreans, Should Be Worried About Mad Cow Tainted USA Beef

By Terry S. Singeltary Sr. May 15, 2008

Straight to the Source

Web Note: This is an important commentary by Terry S. Singeltary Sr., on a recent Business Week story on the controversy in South Korea over their government's lifting on the ban on conventional (non-organic) beef, despite the fact that the USDA is still allowing slaughterhouse waste and blood and manure to be fed to cows, and refusing to test all cows at slaughter. See the Mad Cow section of the OCA website for in-depth information. Terry is a regular blogger on the OCA website on Mad Cow issues.

Ronnie Cummins

One Korean official says the probability of a human being catching a mad cow disease by eating U.S. beef is like the one of a golf player scoring a hole-in-one and then being killed by lightning.

this is typical BSe. you here industry groups comment 'your more likely to get hit by a car than die from CJD'. well, maybe so, but my mother and many more did not die from getting hit by a car, they died from CJD, my mothers being the hvCJD (confirmed), and my neighbors mother died from CJD (confirmed). the UKBSEnvCJD _only_ theory is incorrect. there are more strains of mad cow than the UK BSE in beef to nvCJD in humans in the UK. The deception by the USDA, FDA, and the Bush administration about mad cow disease, CJD, and all Transmissible Spongiform Encephalopathy over the past 8 years have been outrageous, to a point of being criminal. I am vested in nothing, but the truth.

U.K. BSE nvCJD ONLY theory invoked again. it's like still believing the world is flat for pete's sake i.e. the one strain, one country, one age group, one species, one route, only theory. it's pure BSe, and the stench is horrendous. it's the smell of death, for profit only.

THE UKBSEnvCJD _only_ theory is incorrect. there are more strains of mad cow than the UK BSE in beef to nvCJD in humans in the UK. The deception by the USDA, FDA, and the Bush administration about mad cow disease, CJD, and all Transmissible Spongiform Encephalopathy over the past 8 years have been outrageous, to a point of being criminal. I am vested in nothing, but the truth.

snip...

Please remember, the last two mad cows documented in the USA i.e. Alabama and Texas, both were of the 'atypical' BSE strain, and immediately after that, the USDA shut down the testing from 470,000 to 40,000 in the U.S. in 2007 out of about 35 million cattle slaughtered. also, science is showing that some of these atypical cases are more virulent to humans than the typical UK BSE strain ;

***Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.***

Progress Report from the National Prion Disease Pathology Surveillance Center

In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.

IF BSE is not in the USA (just not documented for many different reasons), and only atypical BSE is in the USA (plus CWD, plus, many strains of Scrapie, and Now the Nor-98 documented in 5 different states, plus TME, then why would human mad cow in the USA look like the UK nvCJD from UK BSE cows ? it was shown long ago in studies at Mission Texas that experimental transmission of USA Scrapie to USA Bovine, DID NOT LOOK LIKE UK BSE. so again, in short, why would human mad cow in the USA look like human mad cow in the UK i.e. the (nvCJD). however, I believe that BSE has been in the USA untested and undocumented for years. why on earth then does the USDA refuse to allow creekstone or anyone else test their product? simple, if you don't look/test, you don't find.

snip...

He added that because the CDC only provide information on diseases, they have no plans

to make a separate press release on the issue including the result of the investigation.

and that is the way they plan to keep it, all spontaneous, sporadic, no route, no source $$$

USDA, CDC, NIH, ET AL INVOKE THE UKBSEnvCJD ONLY RULE $$$

Virginia Woman Did not Die of vCJD

Updated Jun.17,2008 08:34 KST

The MBC news program "PD Diary" reported that Aretha Vinson died of variant Creutzfeldt-Jakob Disease (vCJD) in early April when in an interview, Vinson's mother actually said, "The results had come in from the MRI and it appeared that our daughter could possibly have CJD," not vCJD.

The proportion of slaughtered cattle tested for BSE is much smaller in the U.S. than in Europe and Japan, leaving the U.S. heavily dependent on statistical models to estimate both the current prevalence and the spread of BSE. We examine the models relied on by USDA, finding that the prevalence model provides only a rough estimate, due to limited data availability. Reassuring forecasts from the model of the spread of BSE depend on the arbitrary constraint that worst-case values are assumed by only one of 17 key parameters at a time. In three of the six published scenarios with multiple worst-case parameter values, there is at least a 25% probability that BSE will spread rapidly. In public policy terms, reliance on potentially flawed models can be seen as a gamble that no serious BSE outbreak will occur. Statistical modeling at this level of abstraction, with its myriad, compound uncertainties, is no substitute for precautionary policies to protect public health against the threat of epidemics such as BSE.

Believe in what your heart is telling you. Your Countryman and Countrywoman should be proud of your stance to protect your people. Please continue your fight for the truth, as the truth will set us all free. ...TSS

An Arizona meat processing company and its owner pled guilty in February 2007 to charges of theft of Government funds, mail fraud, and wire fraud. The owner and his company defrauded the BSE Surveillance Program when they falsified BSE Surveillance Data Collection Forms and then submitted payment requests to USDA for the services. In addition to the targeted sample population (those cattle that were more than 30 months old or had other risk factors for BSE), the owner submitted to USDA, or caused to be submitted, BSE obex (brain stem) samples from healthy USDA-inspected cattle. As a result, the owner fraudulently received approximately $390,000. Sentencing is scheduled for May 2007.

snip...

Topics that will be covered in ongoing or planned reviews under Goal 1 include:

And last but not least, similarities of PrPres between Htype BSE and human prion diseases like CJD or GSS have been put forward [10], as well as between L-type BSE and CJD [17]. These findings raise questions about the origin and inter species transmission of these prion diseases that were discovered through the BSE active surveillance.

PLEASE NOTE IN REFERENCE TO THE LATEST LONG TERM USDA DOWNER COW SCHOOL LUNCH PROGRAM CASE STUDY FOR VCJD IN CHILDREN

Creutzfeldt-Jakob Disease (Variant) and Bovine Spongiform Encephalopathy (Prion Diseases) Description Since 1996, strong evidence has accumulated for a causal relationship between ongoing outbreaks, primarily in Europe, of a disease in cattle called bovine spongiform encephalopathy (BSE, or “mad cow disease”) and a disease in humans called variant Creutzfeldt-Jakob disease (vCJD). Both disorders, which are caused by an unconventional transmissible agent, are invariably fatal brain diseases with incubation periods typically measured in years (1). Transmission of the BSE agent to humans, leading to vCJD, is believed to occur via ingestion of cattle products contaminated with the BSE agent; the specific foods associated with this transmission are unknown. However, a recently published case-control study involving 132 vCJD cases in the United Kingdom (UK) showed evidence of an increased risk for vCJD associated with the frequency of consuming beef products likely to contain mechanically recovered meat and head meat (such as burgers, meat pies, and sausages) (2). Bioassays and molecular tests have enabled identification of what World Health Organization consultants have classified as “high-infectivity” and “lower infectivity” tissues of cattle with BSE (3). The high-infectivity tissues include the brain, spinal cord, retina, optic nerve, and dorsal root and trigeminal ganglia, suggesting that these tissues can pose a relatively high risk of transmission. The lower infectivity tissues include peripheral nerves (e.g., sciatic and facial nerves), tonsils, nictitating membrane (third eye lid), distal ileum, bone marrow, and possibly thigh muscle. The latter tissue from one cow with BSE transmitted disease to highly BSE-sensitive transgenic mice at a rate indicative of trace levels of infectivity.

NOT to forget the 5 cases of the NOR-98 atypical scrapie documented in the USA in 2007, in five different states. WHICH pathologically looks like some sub-types of sporadic CJD, of which Stanely Prusiner warns of a public health risk ;

***The pathology features of Nor98 in the cerebellum of the affected sheep showed similarities with those of sporadic Creutzfeldt-Jakob disease in humans.

Here we report that both Nor98 and discordant cases, including three sheep homozygous for the resistant PrPARR allele (A136R154R171), efficiently transmitted the disease to transgenic mice expressing ovine PrP, and that they shared unique biological and biochemical features upon propagation in mice. These observations support the view that a truly infectious TSE agent, unrecognized until recently, infects sheep and goat flocks and may have important implications in terms of scrapie control and public health.

Edited by Stanley B. Prusiner, University of California, San Francisco, CA, and approved September 12, 2005 (received for review March 21, 2005)

In FY 2007, 331 scrapie cases have been confirmed and reported by the National Veterinary Services Laboratories (NVSL), including 59* Regulatory Scrapie Slaughter Surveillance (RSSS) cases (Figure 5 and Slide 16). In FY 2007, two field cases, one validation case, and two RSSS cases were consistent with Nor-98 scrapie. The Nor98-like cases originated from flocks in California, Minnesota, Colorado, Wyoming and Indiana respectively. Nineteen cases of scrapie in goats have been reported since 1990 (Figure 6). The last goat case was reported in September 2007.

Millions of North Americans hunt deer and elk (U.S. Department of the Interior, Census Bureau), and there is no doubt that people have been exposed to CWD through venison consumption, particularly in light of recent data showing CWD prions in muscle [2]. Human susceptibility to CWD or to other newly emerging animal TSE [9, 14] is still unclear, although we can be somewhat reassured in that there have been no large scale outbreaks of human TSE cases in Colorado and Wyoming, where CWD has existed for decades [51]. Up until approximately 10 years ago, autopsies were not performed on suspect human TSE cases in many states due to biosafety concerns, therefore the diagnosis of potential new TSE strains has been hampered. This indicates that clinical TSE diagnoses in humans were not confirmed, nor was any strain typing done to look for the appearance of potentially subtle or unusual pathological or biochemical phenotypes of a new TSE strain. Fortunately, the autopsy rate for suspect cases is improving. At the National Prion Disease Pathology Surveillance Center at Case Western Reserve University (Cleveland, Ohio), Creutzfeldt-Jakob disease (CJD) suspect cases are studied and classified by CJD subtype. Thus far,

however there have been no unusual or novel prion subtypes that might indicate the appearance of a new prion strain [7, 41]. Other indirect studies of human susceptibility to CWD also suggest that the risk is low. In biochemical conversion studies, Raymond et al. [68] showed that the efficiency of CWD to convert recombinant human PrP into amyloid fibrils was low, but similar to that of both BSE and scrapie fibrils to do the same. These results suggest that there is a molecular incompatibility in the conversion of human PrPC by CWD, sheep scrapie, or BSE, and that cross species infections in humans may be rare events. To determine whether common PrPSc strain features may link CWD and CJD, histopathology and the PrPSc biochemical characteristics from deer and elk were compared with that of humans with sporadic CJD (sCJD) cases that are methionine homozygous at codon 129 of the Prnp gene by Xie et al. [96], although strain features including histologic profile, target organs, and glycoform patterns will not necessarily remain the same upon crossing species barriers [6, 5, 8, 57]. The PrPSc form is cleaved by proteinase-K (PK) at different sites depending on the conformation of the protein and may aid determination of whether the PrPSc conformation is similar. By western blot (SDS-PAGE) of elk CWD, the unglycosylated PK-resistant PrPSc migrated at 21 kDa, similar to sCJD (MM1 subtype) and the PK cleavage site was the same, occurring at residues 78 and 82 as assessed by N-terminal sequencing. Conformational stability was evaluated by measuring the PrPSc stability under partially denaturing conditions and also showed no significant difference between elk CWD and sCJD MM1 PrPSc. However, elk CWD and human sCJD MM1 strains exhibited distinct glycoform patterns by two dimensional gel electrophoresis, suggesting that the strains differed. Future studies may utilize luminescent conjugated polymers, which were recently shown to distinguish naturally- and experimentally-derived prion strains [79]. To study elk-human prion species barriers, Kong et al. inoculated elk CWD into transgenic mice expressing either human PrP or elk PrP. Whereas the elk PrP expressing mice developed disease after only 118-142 days post-inoculation, human PrP expressing mice (129M) did not develop any features of TSE after more than 657 or more than 756 days [41]. In accordance with these results, Tamgüney et al. also reported that human PrP overexpressing mice were not susceptible to 9 CWD isolates from mule deer, white-tailed deer, and elk [84]. However, mice have a limited lifespan and further passages may be necessary to detect low levels of prion infectivity that may be present subclinically. Although indi rect evidence is accumulating that there may be a robust species barrier for CWD transmission to humans, one report indicates nonhuman primate susceptibility to CWD. Intracerebral inoculation of squirrel monkeys (Saimiri sciureus) demonstrated a positive CWD transmission [49]. Among non-human primates, however, the Prnp sequence of the new world monkeys are the most distant from humans [72], and therefore may not indicate that human prion conversion would occur by CWD.

snip...

11. Disease control challenges posed by CWD

Evidence is building that indicates efficient horizontal transmission occurs in CWD, indeed a complicating aspect in disease control [91]. Potential transmission mechanisms range from spread via direct contact among animals to environmental exposure through grazing in areas contaminated by prion-infected secretions, excretions (saliva, urine, feces), tissues (placenta), or decomposed carcasses. Recently, in a breakthrough finding, saliva from CWD infected deer was shown to transmit prion disease [50]. An additional experiment by Miller and colleagues showed that CWD-infected carcasses allowed to decay naturally in confined pastures can lead to CWD infections in captive deer, demonstrating the potential for environmental contamination to spread infection [55]. Modelling studies have provided further

10

support that environmental contamination is likely playing a significant role in transmitting CWD [56, 53]. Additionally, infectious prions have been demonstrated to bind soil particles and remain infectious to animals by both intracerebral and oral exposure routes [38, 37]. Prion infectivity has been recovered from soil more than two years after experimental exposure to prions, suggesting the soil may serve as a reservoir for CWD prions [75]. Taken together, these results indicate that there may even be multiple sources for CWD exposure, perhaps through direct contact and environmental routes. Significant challenges to CWD eradication exist in free-ranging cervids. Infected deer and elk range over a broad geographic region, and even previously surmised geographic barriers such as the Continental Divide have proven passable by infected animals. Ridding the environment of CWD-contaminated soil or even CWD-infected carcasses is not possible. Moreover, the available ante-mortem diagnostic tests for surveillance are laborious and impractical for large numbers of free-ranging animals [74, 88, 95]. Therefore for a wildlife manager, this disease is costly to survey and difficult to control.

12. Conclusion

CWD in cervids is efficiently transmitted, likely more than any other TSE in animals or humans. Therefore, it is unlikely that this TSE can be eradicated, but perhaps through an improved understanding of transmission routes, biological factors influencing pathogenesis, and the molecular basis of CWD prion conversion, a targeted strategy for interrupting disease spread may be developed.

Acknowledgements

I thank Drs. Michael Miller, Jason Bartz and Mathias Heikenwalder for critical review of the manuscript.

Subject: Interference at the EPA Science and Politics at the U.S. Environmental Protection Agency

Reports and Research

Interference at the EPA

Science and Politics at the U.S. Environmental Protection Agency

The U.S. Environmental Protection Agency (EPA) has the simple yet profound charge "to protect human health and the environment." EPA scientists apply their expertise to protect the public from air and water pollution, clean up hazardous waste, and study emerging threats such as global warming. Because each year brings new and potentially toxic chemicals into our homes and workplaces, because air pollution still threatens our public health, and because environmental challenges are becoming more complex and global, a strong and capable EPA is more important than ever.

Yet challenges from industry lobbyists and some political leaders to the agency's decisions have too often led to the suppression and distortion of the scientific findings underlying those decisions—to the detriment of both science and the health of our nation. While every regulatory agency must balance scientific findings with other considerations, policy makers need access to the highest-quality scientific information to make fully informed decisions.

Concern over this problem led the Union of Concerned Scientists (UCS) to investigate political interference in science at the EPA. The investigation combines dozens of interviews with current and former EPA staff, analysis of government documents, more than 1,600 responses to a survey sent to current EPA scientists, and written comments from EPA scientists.

The results of these investigations show an agency under siege from political pressures. On numerous issues—ranging from mercury pollution to groundwater contamination to climate change—political appointees have edited scientific documents, manipulated scientific assessments, and generally sought to undermine the science behind dozens of EPA regulations. ...

THE only fool is one who fools himself, and GW and his administration and their junk science will fool humans for just so long, then the incubation will catch up. none of this was about science, it was all about commodities and futures and the exporting of beef. nothing else mattered, literally, just ask old stanley prusiner the nobel prize winner for the PRION, what did old stan say ;

In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.

The U.S. Department of Agriculture was quick to assure the public earlier this week that the third case of mad cow disease did not pose a risk to them, but what federal officials have not acknowledged is that this latest case indicates the deadly disease has been circulating in U.S. herds for at least a decade.

The second case, which was detected last year in a Texas cow and which USDA officials were reluctant to verify, was approximately 12 years old.

These two cases (the latest was detected in an Alabama cow) present a picture of the disease having been here for 10 years or so, since it is thought that cows usually contract the disease from contaminated feed they consume as calves. The concern is that humans can contract a fatal, incurable, brain-wasting illness from consuming beef products contaminated with the mad cow pathogen.

"The fact the Texas cow showed up fairly clearly implied the existence of other undetected cases," Dr. Paul Brown, former medical director of the National Institutes of Health's Laboratory for Central Nervous System Studies and an expert on mad cow-like diseases, told United Press International. "The question was, 'How many?' and we still can't answer that."

Brown, who is preparing a scientific paper based on the latest two mad cow cases to estimate the maximum number of infected cows that occurred in the United States, said he has "absolutely no confidence in USDA tests before one year ago" because of the agency's reluctance to retest the Texas cow that initially tested positive.

USDA officials finally retested the cow and confirmed it was infected seven months later, but only at the insistence of the agency's inspector general.

"Everything they did on the Texas cow makes everything USDA did before 2005 suspect," Brown said. ...snip...end

"Actually, Terry, I have been critical of the USDA handling of the mad cow issue for some years, and with Linda Detwiler and others sent lengthy detailed critiques and recommendations to both the USDA and the Canadian Food Agency."

Our prior report identified a number of inherent problems in identifying and testing high-risk cattle. We reported that the challenges in identifying the universe of high-risk cattle, as well as the need to design procedures to obtain an appropriate representation of samples, was critical to the success of the BSE surveillance program. The surveillance program was designed to target nonambulatory cattle, cattle showing signs of CNS disease (including cattle testing negative for rabies), cattle showing signs not inconsistent with BSE, and dead cattle. Although APHIS designed procedures to ensure FSIS condemned cattle were sampled and made a concerted effort for outreach to obtain targeted samples, industry practices not considered in the design of the surveillance program reduced assurance that targeted animals were tested for BSE.

USDA/OIG-A/50601-10-KC Page 27

observe these animals ante mortem when possible to assure the animals from the target population are ultimately sampled and the clinical signs evaluated.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP]

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

Over the next 8-10 weeks, approximately 40% of all the adult mink on the farm died from TME. Since previous incidences of TME were associated with common or shared feeding practices, we obtained a careful history of feed ingredients used over the past 12-18 months. The rancher was a "dead stock" feeder using mostly (>95%) downer or dead dairy cattle and a few horses. Sheep had never been fed.

DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)

The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....

Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!

And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...

Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"

again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.

You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)

The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.